Search Results (13)

Pleuroparenchymal fibroelastosis (PPFE) is characterized by fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. As PPFE appears to occur in patients with heterogeneous etiologies, the disease course is thus also heterogenous, with some patients showing rapid progression while others have slow progression. Therefore, it is very difficult to predict prognosis with PPFE. Needless to say, this problematic matter has influenced the treatment strategy of PPFE patients. In fact, until now no evidence has been shown for use in creating an appropriate management algorithm for PPFE. We speculate that “uncoordinated breathing” is the most important reason for dyspnea in PPFE patients. Because monitoring of physique and not just pulmonary function and radiological evaluation is also very important, particularly in PPFE patients, this review focused on the characteristics of PPFE through an overview of previous studies in this field, and we proposed an algorithm as precision medicine based on the current evidence. Multiple views by the pulmonologist are needed to standardize a clinical algorithm that is necessary to correctly assess PPFE patients under the premise of maintenance of physique by providing appropriate nutritional care and pulmonary rehabilitation. Full article

Idiopathic Interstitial Pneumonias (IIPs) are a heterogeneous group of the broader category of Interstitial Lung Diseases (ILDs), pathologically characterized by the distortion of lung parenchyma by interstitial inflammation and/or fibrosis. The American Thoracic Society (ATS)/European Respiratory Society (ERS) international multidisciplinary consensus classification of the IIPs was published in 2002 and then updated in 2013, with the authors emphasizing the need for a multidisciplinary approach to the diagnosis of IIPs. The histological evaluation of IIPs is challenging, and different types of IIPs are classically associated with specific histopathological patterns. However, morphological overlaps can be observed, and the same histopathological features can be seen in totally different clinical settings. Therefore, the pathologist’s aim is to recognize the pathologic–morphologic pattern of disease in this clinical setting, and only after multi-disciplinary evaluation, if there is concordance between clinical and radiological findings, a definitive diagnosis of specific IIP can be established, allowing the optimal clinical–therapeutic management of the patient. Full article

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterized by fibrosis involving the pleura and the upper lobes which can be idiopathic or secondary to chemotherapy, transplantations and occupational exposure. For the end-stage form, lung transplantation (LT) is the treatment of choice. The aim of this study was to report our single-center experience for patients subjected to LT for PPFE and comparing it with the already published evidence on this topic. At our center, we have performed 6 bilateral LTs for patients with PPFE (3 males and 3 females) with a median age of 52 years. Median ICU and in-hospital length of stay were 8 and 30 days, respectively. To date, two patients are alive and four are dead, with a median overall survival of 10 months. In addition, after a formal search using the terms “pleuroparenchymal fibroelastosis AND lung transplantation”, we collected 14 studies focused on outcomes after LT. LT for PPFE is technically challenging and its post-operative course could also be complicated. Current available data on LT outcomes are extremely poor and mostly limited to case reports. Further studies need to be published to improve knowledge of this disease and to achieve best outcomes for LT. Full article

Background: Pleuroparenchymal Fibroelastosis (PPFE) is a rare disease that consists of elastofibrosis that involves the pleura and subpleural lung parenchyma; it is an unusual pulmonary disease with unique clinical, radiological and pathological characteristics. According to recent studies, PPFE may not be a definite disease but a form of chronic lung injury. The aim of this retrospective study is to determine the incidence and to evaluate the distribution, severity and progression of this radiological entity on high-resolution CT (HRCT) exams of the chest, performed in routine clinical practice. In total, 1514 HRCT exams performed in the period January 2016–June 2018 were analyzed. For each exam, the presence of PPFE was evaluated and a quantitative score was assigned (from 0 to 7 points, based on the maximum depth of fibrotic involvement of the parenchyma). When available, two exams with a time interval of at least 6 months were compared for each patient in order to evaluate progression (defined as the increase in the disease score). Patients were divided into different groups according to exposure and their associated diseases. Statistical analysis was performed by using the Wilcoxon test and Kruskal–Wallis test. Results: PPFE was detected in 174 out of 1514 patients (11.6%), with a mean score of 6.1 ± 3.9 (range 1–14). In 106 out of 174 patients (60.9%), a previous CT scan was available and an evolution of PPFE was detected in 19 of these (11.5%). Among these 19 patients with worsening PPFE, 4 had isolated PPFE that was associated with chronic exposure or connective tissue disorders, and the other 15 had an associated lung disease and/or a chronic exposure. In this group, it was found that the ventral segments of the upper lobes, fissures and apical segments of the lower lobes had a greater statistically significant involvement in the progression of the disease compared to the non-progressive group. In 16 of 174 patients (9.2%, 7 of which belonged to the radiological progression group) a biopsy through video-assisted thoracoscopic surgery or apicoectomy confirmed PPFE. Conclusion: PPFE-like lesions are not uncommon on HRCT exams in routine clinical practice, and are frequently found in patients with different forms of chronic lung injury. Further studies are necessary to explain why the disease progresses in some cases, while in most, it remains stationary over time. Full article

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that is currently classified as an idiopathic interstitial pneumonia. Although originally described as an idiopathic disease, PPFE has now been identified as a rare complication following hematopoietic stem cell transplant (HSCT). Unlike other pulmonary complications after HSCT, PPFE occurs very late after transplant. Etiologies for PPFE after HSCT remain to be fully established. Infections and adverse effects to alkylating chemotherapy have been suggested as possible causes. In several cases, there is an association of PPFE with bronchiolitis obliterans syndrome after HSCT, suggesting that PPFE may be another manifestation of pulmonary chronic graft versus host disease after HSCT. Algorithms have been designed to assist in confirming a diagnosis of PPFE without the need for a surgical lung biopsy, however at present, no biomarker is established for the diagnosis or to predict the progression of disease. Presently, there is no current therapy for PPFE, but fortunately the disease progresses slowly in most patients. Full article

Background: In idiopathic pulmonary fibrosis (IPF), 6-minute walking distance (6MWD) is an independent factor for mortality. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease with physical features such as emaciation, but the relationship between IPPFE and 6MWD is unclear. In this study, we investigated the factors that cause a decrease in the percent of the predicted value of a 6-minute walk distance (%6MWD), including the disease entities, IPF and IPPFE. Methods: This study included 100 patients (IPF: 75 patients, IPPFE: 25 patients, age: 73.5 ± 7.2 years, sex: 73 males) who visited the rehabilitation department. Patients with a %6MWD ≥ 80% were assigned to the normal group (n = 54), and patients with a %6MWD < 80% were assigned to the decreased group (n = 46). The items showing a significant difference between groups were used as independent variables, after the consideration of multicollinearity, for a logistic analysis where %6MWD < 80% was used as the dependent variable. Results: The 6MWD results show that there was no significant difference between IPF and IPPFE in the absolute value of 6MWD and in the number of people with 6MWD ≥ 250 m, but when 6MWD was compared with %6MWD, the IPPFE group showed a significantly lower value than the IPF group (p = 0.013). Logistic regression analysis showed that only BMI (p = 0.032), GAP index (p = 0.043), and mMRC (p = 0.026) were factors that caused a decrease in %6MWD in 100 patients. Conclusion: The results suggest that leanness, shortness of breath and severity of illness are the most important factors that determine exercise tolerance, regardless of disease entity in IPF and IPPFE. Full article

Pulmonary fibroelastotic remodelling occurs within a broad spectrum of diseases with vastly divergent outcomes. So far, no comprehensive terminology has been established to adequately address and distinguish histomorphological and clinical entities. We aimed to describe the range of fibroelastotic changes and define stringent histological criteria. Furthermore, we wanted to clarify the corresponding terminology in order to distinguish clinically relevant variants of pulmonary fibroelastotic remodelling. We revisited pulmonary specimens with fibroelastotic remodelling sampled during the last ten years at a large European lung transplant centre. Consensus-based definitions of specific variants of fibroelastotic changes were developed on the basis of well-defined cases and applied. Systematic evaluation was performed in a steps-wise algorithm, first identifying the fulcrum of the respective lesions, and then assessing the morphological changes, their distribution and the features of the adjacent parenchyma. We defined typical alveolar fibro-elastosis as collagenous effacement of the alveolar spaces with accompanying hyper-elastosis of the remodelled and paucicellular alveolar walls, independent of the underlying disease in 45 cases. Clinically, this pattern could be seen in (idiopathic) pleuroparenchymal fibro-elastosis, interstitial lung disease with concomitant alveolar fibro-elastosis, following hematopoietic stem cell and lung transplantation, autoimmune disease, radio-/chemotherapy, and pulmonary apical caps. Novel in-transit and activity stages of fibroelastotic remodelling were identified. For the first time, we present a comprehensive definition of fibroelastotic remodelling, its anatomic distribution, and clinical associations, thereby providing a basis for stringent patient stratification and prediction of outcome. Full article

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinctive interstitial pneumonia with upper lobe predominance that shows unique morphological features among idiopathic interstitial pneumonias (IIPs). Affected patients have a variety of clinical presentations with heterogeneous clinical courses. Cluster analysis is a valuable tool for identifying distinct clinical phenotypes under heterogeneous conditions. This study aimed to identify the phenotypes of patients with idiopathic PPFE. Using cluster analysis, novel PPFE phenotypes were identified among subjects from our multicenter cohort, and outcomes were stratified according to phenotypic clusters. Among the subjects with baseline data (N = 84), four clusters were identified. Cluster 1 included younger male subjects with coexisting non-UIP-like patterns. Cluster 2 included elderly female nonsmokers with low body mass index (BMI). Cluster 3 included elderly male smokers with a coexisting IP-like pattern. Cluster 4 included younger male smokers without lower lobe lesions. Patients in cluster 3 had significantly worse survival outcomes than those in clusters 1, 2, and 4 (p < 0.001, p = 0.0041, and p = 0.0155, respectively). Among idiopathic PPFE patients, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might predict survival outcomes. Full article

Background: Bronchiolitis obliterans syndrome (BOS) and restrictive allograft syndrome (RAS) are two distinct phenotypes of chronic lung allograft dysfunction (CLAD) in lung transplant (LTx) recipients. Contrary to BOS, RAS can radiologically present with a pleuroparenchymal fibroelastosis (PPFE) pattern. This study investigates lung ultrasound (LUS) to identify potential surrogate markers of PPFE in order to distinguish CLAD phenotype RAS from BOS. Methods: A prospective cohort study performed at a National Lung Transplantation Center during June 2016 to December 2017. Patients were examined with LUS and high-resolution computed tomography of the thorax (HRCT). Results: Twenty-five CLAD patients (72% males, median age of 54 years) were included, corresponding to 19/6 BOS/RAS patients. LUS-identified pleural thickening was more pronounced in RAS vs. BOS patients (5.6 vs. 2.9 mm) compatible with PPFE on HRCT. LUS-identified pleural thickening as an indicator of PPFE in RAS patients’ upper lobes showed a sensitivity of 100% (95% CI; 54–100%), specificity of 100% (95% CI; 82–100%), PPV of 100% (95% CI; 54–100%), and NPV of 100% (95% CI; 82–100%). Conclusion: Apical pleural thickening detected by LUS and compatible with PPFE on HRCT separates RAS from BOS in patients with CLAD. We propose LUS as a supplementary tool for initial CLAD phenotyping. Full article

Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the prevalence and incidence, clinical and pathological characteristics, and disease course of PPFE. Patients with PPFE reportedly have several unique clinical characteristics—including an extremely low body mass index with a slender body and chest wall deformity, known as “flat chest”. As this disease progresses, shrinking of the lungs often causes life-threatening complications, such as pneumothorax, and associated air leak syndrome. Lung transplantation is considered the only effective treatment for patients with advanced PPFE; however, little is known about the influences of the characteristics of PPFE on the outcome of lung transplantation. This review focuses on the unique clinicopathologic characteristics of PPFE and associated outcomes of lung transplantation for these patients. Full article

The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED and the features of the denudated areas in 26 PPFE cases, 30 idiopathic pulmonary fibrosis (IPF) cases, and 29 controls. PPFE patients had lower forced vital capacities and higher residual volume/total lung capacities in pulmonary function tests compared to IPF and control patients. Histopathologically, subpleural fibroelastosis was observed in PPFE, and AED was observed in 12.01% of cases in the subpleural or interlobular septa regardless of fibroelastosis. The percentage of AED in the PPFE group was significantly higher than that in the IPF group (6.84%; p = 0.03) and the normal group (1.19%; p < 0.001). In the IPF group, the percentage of AED and the presence of PPFE-like lesions in the upper lobes were examined radiologically, but no correlation was found. We showed that AED frequently occurred in PPFE. AED was less frequent in IPF, which, in combination with imaging data, suggests that PPFE may have a different pathogenesis from IPF. Full article

Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary and idiopathic PPFE. Methods: We retrospectively reviewed the medical records of consecutive PPFE patients between January 1999 and December 2018. We identified 132 idiopathic PPFE patients and 32 secondary PPFE patients. Results: The incidence of interstitial lung disease (ILD) pattern different from the usual interstitial pneumonia (UIP) pattern in the lower lobes was higher in secondary PPFE patients (38.5%) than in idiopathic PPFE patients (61.5%, p = 0.02). The idiopathic and secondary PPFE groups did not differ significantly in terms of laboratory data, respiratory complications, and survival (median: 5.0 years vs. 4.1 years, p = 0.95). The presence of UIP pattern was independently associated with increased mortality in multivariate analyses in idiopathic PPFE patients, but not in secondary PPFE patients. Conclusions: The frequency and prognostic impact of UIP-pattern ILD differed between idiopathic and secondary PPFE patients. Lung transplantation should be considered in secondary PPFE patients with low diffusing capacity of the lungs for carbon monoxide (DLCO) regardless of lower-lobe ILD pattern. Full article

Background: Diagnostic criteria of idiopathic pleuroparenchymal fibroelastosis (IPPFE) were recently proposed, including physiological criteria of the body mass index (BMI) and percentage of the predicted values of residual volume (RV)/total lung capacity (TLC) (RV/TLC %pred.). The aim of this study was to evaluate (i) whether the physiologic criteria are useful for the diagnosis and (ii) whether the flat chest index, defined as the ratio of the anteroposterior diameter to the transverse diameter of the thoracic cage, could be an alternative parameter to RV/TLC %pred. Methods: We selected consecutive IPPFE patients and idiopathic pulmonary fibrosis (IPF) patients. We examined the diagnostic sensitivity and specificity of the physiological criteria and flat chest index for differentiating IPPFE patients from IPF patients. Results: This study included 37 IPPFE patients and 89 IPF patients. The physiological criteria distinguished IPPFE patients from IPF patients with a sensitivity of 78.6% and specificity of 88.0%. The combination of the flat chest index and BMI was also effective for differentiation (sensitivity of 82.1% and specificity of 89.3%). Conclusion: We verified the good performance of the physiologic criteria in a different cohort. When the RV/TLC is not measured, using the flat chest index instead of RV/TLC %pred. may be reasonable. Full article